Peripheral Nerve Disorders 2

Author: Arthur K. Asbury
Publisher: Butterworth-Heinemann Medical
ISBN:
Size: 32.27 MB
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Peripheral Nerve Disorders 2 from the Author: Arthur K. Asbury. The flow of new information in the eleven years since the publication of the first volume (published in the BIMR series), has long necessitated a new look at peripheral nerve disorders. Thus this new volume emphasises the advances of the past decade and includes information on newly recognized neuropathies, hereditary neuropathies, peripheral nerve dysfunction and therapeutic advances. It has over 20 contributors from the UK, USA and continental Europe.

Peripheral Nerve Disorders

Author: Simon K.W. Lloyd
Publisher: Elsevier Inc. Chapters
ISBN: 0128078855
Size: 79.56 MB
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Peripheral Nerve Disorders from the Author: Simon K.W. Lloyd. Neurofibromatosis type 2 (NF2) is an autosomal dominant inherited tumor predisposition syndrome caused by mutations in the NF2 gene on chromosome 22. Affected individuals develop schwannomas typically involving both vestibular nerves leading to hearing loss and eventual deafness. Rehabilitation with brainstem implants and in some cases cochlear implants is improving this outcome. Schwannomas also occur on other cranial nerves, on spinal nerve roots and peripheral nerves, and intracutaneously as plaques. Cranial and spinal meningiomas and spinal ependymomas are other common tumors. Fifty to sixty percent of patients represent de novo mutations and as many as 33% of these are mosaic for the underlying disease causing mutation. Truncating mutations (nonsense, frameshift insertions/deletions) are the most frequent germline events and cause the most severe disease, whilst single and multiple exon deletions are common and are usually associated with milder NF2. Neurological deficits are a major feature of the condition and neurologists have a pivotal role in assigning symptoms to lesions and in managing neuropathies. NF2 represents a difficult management problem with most patients facing substantial morbidity and reduced life expectancy. Surgery remains the focus of current management although watchful waiting and occasionally radiation treatment have a role. We are seeing the advent of tailored drug therapies aimed at the genetic level and these are likely to provide huge improvements for this devastating, life-limiting condition.

Peripheral Nerve Disorders

Author: Gérard Said
Publisher: Elsevier Inc. Chapters
ISBN: 0128078448
Size: 17.21 MB
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Peripheral Nerve Disorders from the Author: Gérard Said. Examination of a patient with peripheral neuropathy starts with careful questioning of the patient about the history of symptoms and signs and of a possible familial disorder. Several steps are required during examination of the patient with peripheral neuropathy: first the pattern of neuropathy and site of lesions should be determined: roots, nerve trunks, focal, multifocal, length-dependent generalized polyneuropathy, the type of nerve fibers predominantly affected, the association with trophic changes and autonomic dysfunction, the course of the disease ranging from acute inflammatory polyneuritis or fulminant multifocal neuropathy to an extremely slow progression as in Charcot−Marie−Tooth syndromes. At the end of this first contact with the patient, the neurologist must decide which investigations seem necessary and their timing including electrophysiological tests, imaging, CSF examination, blood tests, nerve and muscle biopsy, DNA testing, etc. In some cases, life-threatening manifestations, including weakness of respiratory muscles or swallowing difficulty, or autonomic dysfunction, require urgent therapeutic decisions.

Peripheral Nerve Disorders

Author: Israel Steiner
Publisher: Elsevier Inc. Chapters
ISBN: 0128078626
Size: 25.96 MB
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Peripheral Nerve Disorders from the Author: Israel Steiner. Among the human herpes viruses, three are neurotropic and capable of producing severe neurological abnormalities: herpes simplex virus type 1 and 2 (HSV-1 and HSV-2) and varicella-zoster virus (VZV). Both the acute, primary infection and the reactivation from the site of latent infection, the dorsal sensory ganglia, are associated with severe human morbidity and mortality. The peripheral nervous system is one of the major loci affected by these viruses. The present review details the virology and molecular biology underlying the human infection. This is followed by detailed description of the symtomatology, clinical presentation, diagnosis, course, therapy, and prognosis of disorders of the peripheral nervous system caused by these viruses.

Peripheral Nerve Disorders

Author: Jean-Michel Vallat
Publisher: John Wiley & Sons
ISBN: 1118618432
Size: 74.18 MB
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Peripheral Nerve Disorders from the Author: Jean-Michel Vallat. Peripheral Nerve Disorders: Pathology and Genetics is a definitive, clinically-oriented guide to the pathology of peripheral nerve disorders. These commonly seen neurological challenges have many causes and accurate diagnosis is often necessary via pathological analysis. New techniques exploiting molecular biological knowledge have opened up new vistas to understanding the pathogenesis of these disorders, and hence their effective management. This new title takes a disease-oriented approach to understanding the pathology of these conditions. It combines classical and contemporary techniques to enable practitioners in neurology and neuropathology to better understanding of the disease processes underlying patients’ presentations and to formulate appropriate management plans. Peripheral Nerve Disorders: Pathology and Genetics is a valuable resource for neurologists, neuropathologists, pathologists, neurobiologists and geneticists.

Peripheral Nerve Disorders

Author: Kurien Koshy
Publisher: Elsevier Inc. Chapters
ISBN: 0128078758
Size: 12.19 MB
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Peripheral Nerve Disorders from the Author: Kurien Koshy. Patients admitted to intensive care units (ICUs) suffer from a wide range of neurological disorders. Some develop within the ICU rendering weakness and difficulty in weaning patients from ventilator support. ICUAW, or ICU acquired weakness, is a broad term that includes several more specific neuromuscular problems. After exclusion of other causes of weakness, ICUAW includes critical illness polyneuropathy (CIP), first described by Charles Bolton, critical illness myopathy (CIM), and disorders of neuromuscular junction transmission. This chapter reviews the clinical, electrophysiological, and pathological features of these conditions and provides clinicians with approaches toward diagnosing and investigating ICUAW.

Diagnosis And Management Of Peripheral Nerve Disorders

Author: Jerry R. Mendell
Publisher: Oxford University Press
ISBN: 9780198030485
Size: 10.73 MB
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Diagnosis And Management Of Peripheral Nerve Disorders from the Author: Jerry R. Mendell. Peripheral neuropathies represent a challenging subject for most physicians. This is an up-to-date, comprehensive, and readable book on peripheral neuropathies that includes concise information on the clinical, electrophysiological, pathological, pathogenic, and treatment aspects of the most important disorders. New molecular and serologic diagnostic tests are discussed. Sections are devoted to nerve and skin biopsy techniques and findings, quantitative sensory and autonomic reflex tests. Case examples are used liberally throughout the text. The editors: Mendell, Kissel, and Cornblath are experienced clinicians that bring complementary knowledge to each of the subjects. Additional authors have been handpicked for specific topics which add to the value of the edition.

Peripheral Nerve Disorders

Author: Sinéad M. Murphy
Publisher: Elsevier Inc. Chapters
ISBN: 012807843X
Size: 56.34 MB
Format: PDF
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Peripheral Nerve Disorders from the Author: Sinéad M. Murphy. The inherited neuropathies are a clinically and genetically heterogeneous group of disorders in which there have been rapid advances in the last two decades. Molecular genetic testing is now an integral part of the evaluation of patients with inherited neuropathies. In this chapter we describe the genes responsible for the primary inherited neuropathies. We briefly discuss the clinical phenotype of each of the known inherited neuropathy subgroups, describe algorithms for molecular genetic testing of affected patients and discuss genetic counseling. The basic principles of careful phenotyping, documenting an accurate family history, and testing the available genes in an appropriate manner should identify the vast majority of individuals with CMT1 and many of those with CMT2. In this chapter we also describe the current methods of genetic testing. As advances are made in molecular genetic technologies and improvements are made in bioinformatics, it is likely that the current time-consuming methods of DNA sequencing will give way to quicker and more efficient high-throughput methods, which are briefly discussed here.

Peripheral Nerve Disorders

Author: Davide Pareyson
Publisher: Elsevier Inc. Chapters
ISBN: 0128078782
Size: 12.42 MB
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Peripheral Nerve Disorders from the Author: Davide Pareyson. Charcot–Marie–Tooth neuropathy (CMT) is a group of genetically heterogeneous disorders sharing a similar phenotype, characterized by wasting and weakness mainly involving the distal muscles of lower and upper limbs, variably associated with distal sensory loss and skeletal deformities. This chapter deals with dominantly transmitted CMT and related disorders, namely hereditary neuropathy with liability to pressure palsies (HNPP) and hereditary neuralgic amyotrophy (HNA). During the last 20 years, several genes have been uncovered associated with CMT and our understanding of the underlying molecular mechanisms has greatly improved. Consequently, a precise genetic diagnosis is now possible in the majority of cases, thus allowing proper genetic counseling. Although, unfortunately, treatment is still unavailable for all types of CMT, several cellular and animal models have been developed and some compounds have proved effective in these models. The first trials with ascorbic acid in CMT type 1A have been completed and, although negative, are providing relevant information on disease course and on how to prepare for future trials.

Peripheral Nerve Disorders

Author: Andreas Steck
Publisher: Elsevier Inc. Chapters
ISBN: 0128078421
Size: 25.88 MB
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Peripheral Nerve Disorders from the Author: Andreas Steck. The identification of autoantibodies associated with dysimmune neuropathies was a major contribution to the characterization of peripheral nerve disorders, the understanding of their pathophysiology, and the clinical diagnosis of neuropathies. Antibodies directed to GM1, GQ1b, and disyalilated gangliosides, and anti-MAG antibodies are very useful in the diagnosis of acute or chronic motor or sensory-motor neuropathies with or without monoclonal IgM. Anti-onconeural anti-Hu and anti-CV2/CRMP antibodies allow when they are detected the diagnosis of paraneoplastic neuropathies. This chapter focuses on the description of these antibodies as diagnostic markers and on their immunopathogenesis. We give a background overview on the origin of these antibodies, their detection, and review those studies, which clearly show that these antibodies are capable of binding to the target tissues in peripheral nerve and thereby can exert a variety of pathophysiological effects. The corresponding electrophysiological and histological changes observed both in human and animal models are exemplified in order to get a better understanding of the immune mechanisms of these antibody-mediated neuropathies.